A rare cause of the small bowel ulcers
Václav Leksa1, Josef Kubát1, Petr Vyhnálek1
1 Interní oddělení, Pardubická nemocnice, Nemocnice Pardubického kraje, a. s.
Zollinger-Ellison syndrome is a very rare disease characterized by increased production of gastric acid that leads to peptic ulcer disease and diarrhea. It is caused by disproportionate ectopic secretion of gastrin from the cells of the endocrine tumor of digestive system – gastrinoma. It occurs sporadically or as a part of multiple endocrine neoplasia type 1. The tumor is localized mostly in duodenum and pancreas. The treatment combines control of gastric acid secretion, nowadays mainly by proton pump inhibitors and the treatment of gastrinoma itself – surgical or oncological (chemotherapy, biological treatment). In our paper, we present a case of a middle-aged woman examined for abdominal pain and diarrhea. Her initial gastroscopy showed erosive duodenitis; we excluded the infectious etiology of the diarrhea. During the examination process, there were severe complications of peptic ulcer disease for which several surgeries were done. From all the laboratory results and imaging methods, the final diagnosis was made – Zollinger-Ellison syndrome due to gastrinoma of the tail of pancreas and the patient underwent surgery. Unfortunately, there was a progression of the disease in a short time that requested another oncological and surgical treatment.
Keywordsproton pump inhibitors, peptický vřed, Zollingerův-Ellisonův syndrom, gastrinom, gastrin
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1. Zollinger RM, Ellison EH. Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann Surg 1955; 142 (4): 709–728.
2. Epelboym I, Mazeh H. Zollinger-Ellison syndrome: classical considerations and current controversies. Oncologist 2014; 19 (1): 44–50. doi: 10.1634/theoncologist.2013-0369.
3. Weber HC, Venzon DJ, Lin JT et al. Determinants of metastatic rate and survival in patients with Zollinger-Ellison syndrome: a prospective long-term study. Gastroenterology 1995; 108 (6): 1637–1649. doi: 10.1016/0016-5085 (95) 90124-8.
4. Klimstra DS, Kloppell G, La Rosa S et al. Classification of neuroendocrine neoplasms of the digestive system. In: WHO Classification of Tumours Editorial (ed). Digestive system tumours: who classification of tumours. 5th ed. Lyon: World Heatlh Orgn 2019: 16.
5. Norton JA, Foster DS, Ito T et al. Gastrinomas: medical or surgical treatment. Endocrinol Metab Clin North Am 2018; 47 (3): 577–601. doi: 10.1016/j.ecl.2018.04.009.
6. Berna MJ, Hoffmann KM, Serrano J et al. Serum gastrin in Zollinger-Ellison syndrome: I. prospective study of fasting serum gastrin in 309 patients from the National Institutes of Health and comparison with 2229 cases from the literature. Medicine (Baltimore) 2006; 85 (6): 295–330. doi: 10.1097/01.md.0000236956.74128.76.
7. Roy PK, Venzon DJ, Shojamanesh H et al. Zollinger-Ellison syndrome. Clinical presentation in 261 patients. Medicine (Baltimore) 2000; 79 (6): 379–411. doi: 10.1097/00005792-200011000-00004.
8. Metz DC, Cadiot G, Poitras P et al. Diagnosis of Zollinger-Ellison syndrome in the era of PPIs, faulty gastrin assays, sensitive imaging and limited access to acid secretory testing. Int J Endocr Oncol 2017; 4 (4): 167–185. doi: 10.2217/ije-2017-0018.
9. Tomassetti P, Migliori M, Simoni P et al. Diagnostic value of plasma chromogranin A in neuroendocrine tumours. Eur J Gastroenterol Hepatol 2001; 13 (1): 55–58. doi: 10.1097/000 42737-200101000-00010.
10. Jensen RT, Cadiot G, Brandi ML et al. ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes. Neuroendocrinology 2012; 95 (2): 98–119. doi: 10.1159/000335591.
11. Deppen SA, Liu E, Blume JD et al. Safety and efficacy of 68Ga-DOTATATE PET/CT for diagnosis, staging, and treatment management of neuroendocrine tumors. J Nucl Med 2016; 57 (5): 708–714. doi: 10.2967/jnumed.115.163865.
12. Ito T, Igarashi H, Jensen RT. Therapy of metastatic pancreatic neuroendocrine tumors (pNETs): recent insights and advances. J Gastroenterol 2012; 47 (9): 941–960. doi: 10.1007/s00535-012-0642-8.